Onset and pre-onset studies to define the Huntington's disease natural history

F. Squitieri, M. Cannella, P. Giallonardo, V. Maglione, C. Mariotti, M. R. Hayden

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

Huntington's disease's (HD) clinical history has not been defined yet. However, many aspects of the most confusing clinical stages, i.e., the first and last disease phases, including the symptom progression and the disease duration, have been better approached after discovery of the responsible gene. The existence of accurate genetic tests, available for affected and pre-symptomatic subjects (i.e., mutation carriers) and the possibility to study transgenic in vivo models, are actually helping us to understand some of the aspects of HD clinical presentation. HD may present with motor symptoms other than chorea, the psychiatric manifestations may represent part of the clinical picture and cognitive deterioration may occur very early in the disease and depend on early cortical involvement. Pre-onset studies are of crucial importance in understanding the temporal sequence of the clinical events. This is also very important for future therapeutic strategies in those diseases initiating late in the life, such as HD.

Original languageEnglish
Pages (from-to)233-238
Number of pages6
JournalBrain Research Bulletin
Volume56
Issue number3-4
DOIs
Publication statusPublished - Nov 1 2001

Fingerprint

Huntington Disease
Natural History
Chorea
Genetic Association Studies
Psychiatry
Disease Progression
Mutation
Therapeutics

Keywords

  • Clinical history
  • Onset symptoms
  • Predictive diagnosis

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Onset and pre-onset studies to define the Huntington's disease natural history. / Squitieri, F.; Cannella, M.; Giallonardo, P.; Maglione, V.; Mariotti, C.; Hayden, M. R.

In: Brain Research Bulletin, Vol. 56, No. 3-4, 01.11.2001, p. 233-238.

Research output: Contribution to journalArticle

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